Abstract
Background:
Pulmonary atresia with intact ventricular septum (PAIVS) may be unable to compensate for right heart failure in the remote period due to inadequate right ventricular function, even if both ventricular circulations are maintained in the short term. One-and-a-half repairs may have a lower risk of remote right heart failure and severe complications than biventricular repair (BVR). We compared complications and hemodynamics during the medium term for each type of circulatory repair, estimated the risk of right heart failure, and discussed the PAIVS strategy, considering the remote prognosis.
Materials and Methods:
Of 105 PAIVS patients who underwent surgical intervention at our hospital, we excluded patients who died (n = 8) and those with Glenn circulation awaiting Fontan repair (n = 3). Ninety-four patients were analyzed (BVR, n = 16; one-and-a-half, n = 6; Fontan, n = 72). Hemodynamics and complications in the medium term were compared among groups according to the final repair.
Results:
The mean age (range) at the last evaluation was as follows: BVR, 17.5 (2–39) years; one-and-a-half, 28.5 (6–34) years; and Fontan, 14 (3–36) years. The New York Heart Association (NYHA) classification was lowest in the one-and-a-half group (P = 0.06) (NYHA I/II/III: BVR, 11/5/0; one-and-a-half, 6/0/0; Fontan, 65/7/0). The central venous pressure was the lowest in the one-and-a-half group: one-and-a-half, 4 (1–14) mmHg; BVR, 4.5 (1–10) mmHg; and Fontan, 8 [5–15] mmHg.
Conclusions:
A one-and-a-half repair is a final repair because it offers a safer hemodynamic margin and may also carry a lower risk of long-term right ventricle failure than BVR.
Keywords: Biventricular repair, Fontan operation, one-and-a-half repair, single ventricle palliation
INTRODUCTION
Pulmonary atresia with intact ventricular septum (PAIVS) is a congenital heart disease characterized by right ventricle (RV) hypoplasia. In severe cases, univentricular repair is inevitable due to marked RV hypoplasia. However, biventricular repair (BVR) may be feasible when the RV has an adequate volume. Although biventricular circulation can be achieved in the short term, it is impossible to compensate for right heart failure in the long term because of an insufficient RV function, and some patients require conversion from BVR to one-and-a-half repair or one-and-a-half repair to Fontan repair.[1,2,3]
Considering the potential long-term risk of RV failure and related complications in adulthood, surgical strategies should ideally be determined with remote-phase outcomes in mind. Nevertheless, few studies have evaluated the mid-to long-term outcomes across all three repairs. We sought to assess and clarify the clinical significance and appropriate role of a one-and-a-half repair by comparing the mid-term outcomes and hemodynamics following BVR, a one-and-a-half repair, and Fontan repair.
MATERIALS AND METHODS
Excluding eight deceased patients and three patients currently at the Glenn stage awaiting Fontan completion, we analyzed 94 PAIVS patients treated at our institution. The patients were divided into three groups (BVR, one-and-a-half, and Fontan) based on their final surgical treatment. We compared mid-term complications and hemodynamic parameters among the three groups [Figure 1]. In addition, we reviewed the causes of death in eight PAIVS patients.
Figure 1.
Flow chart of patient selection in this study. BVR: Biventricular repair, PAIVS: Pulmonary atresia with intact ventricular septum
This study was approved by the Ethics Committee of Fukuoka Children’s Hospital (Approval No. 2022-81), and informed consent was obtained using an opt-out approach.
Hemodynamic parameters and clinical and laboratory data were retrospectively collected from medical records. Hemodynamic parameters were obtained using cardiac catheterization (superior vena cava pressure [SVCP], inferior vena cava pressure [central venous pressure (CVP)], ascending aortic pressure [systolic and diastolic], left ventricular end-diastolic pressure, pulmonary artery wedge pressure [PAWP], and blood gas parameters (alveolar-arterial oxygen difference [AaDO₂], partial pressure of arterial carbon dioxide, partial pressure of arterial oxygen [PaO₂], arterial oxygen saturation [SaO₂]) cardiopulmonary exercise testing (peak oxygen consumption [peak VO₂]), cardiac magnetic resonance imaging (magnetic resonance imaging [MRI]: right ventricular end-diastolic volume [RVEDV], right ventricular ejection fraction [RVEF], left ventricular end-diastolic volume, and left ventricular ejection fraction), and transthoracic echocardiography (severity of tricuspid regurgitation [TR]; graded as follows: grade 1, trivial; Grade 2, mild; Grade 3, moderate; Grade 4, severe).
Cardiac MRI was performed on a 1.5-Tesla MRI scanner (Avanto Magneton; Siemens®) at atmospheric pressure and with electrocardiographic gating. An image analysis was conducted using the CVI42 software program (Circle Cardiovascular Imaging Inc., Calgary, Canada).
For statistical analyses, the Kruskal–Wallis test was used for inter-group comparisons, with Bonferroni correction applied for post hoc multiple comparisons. The Chi-square test was used to compare categorical variables among the groups. Pearson’s correlation coefficient was used to evaluate the relationships between continuous variables. Freedom from reoperation for each repair type was assessed using a Kaplan–Meier analysis. All statistical analyses were performed using EZR.[4] P value < 0.05 was considered statistically significant.
RESULTS
The cohort was classified into the BVR (n = 16), one-and-a-half (n = 6), and Fontan (n = 72) groups [Table 1]. While some cases were converted intraoperatively from BVR to one-and-a-half repair, none underwent conversion from BVR to one-and-a-half repair, or from one-and-a-half repair to Fontan repair, in the late postoperative period.
Table 1.
Characteristics of patients with pulmonary atresia with intact ventricular septum
| Characteristics | BVR (n=16) | One-and-a-half (n=6) | Fontan (n=72) | P |
|---|---|---|---|---|
| Age (years) | 17.5 (2–39) | 28.5 (6–34) | 14 (3–36) | 0.024 |
| Sex | ||||
| Male | 12 | 3 | 40 | 0.309 |
| Female | 4 | 3 | 32 | |
| SaO2 (%) | 97.5 (83.5–98.5) | 96 (93–97) | 95.1 (91.2–97.4) | 0.035e-2 |
| CVP (mmHg) | 4.5 (1–10) | 4 (1–14) | 8 (5–15) | 0.187e-5 |
| SVCP (mmHg) | 5 (1–10) | 10 (7–15) | 8 (5–14) | 0.965e-6 |
| AscAo pressure (systole) (mmHg) | 94 (72–118) | 96 (82–123) | 95 (71–131) | 0.704 |
| AscAo pressure (diastole) (mmHg) | 57 (40–84) | 61 (47–71) | 58 (39–91) | 0.687 |
| LVEDP (mmHg) | 7 (6–12) | 7 (5–13) | 6 (2–14) | 0.088 |
| PAWP (mmHg) | 5 (1–11) | 5 (1–9) | 2 (1–9) | 0.951e-3 |
| RVEDV (catheter) (percentage of normal) | 104.6 (50.6–176.5) | 102 (21.3–166.8) | 14.5 (5.0–28.6) | 0.416e-5 |
| RVEDV (MRI) (percentage of normal) | 111 (63–184) | 60.5 (26–117) | 9 (2–61) | 0.563e-9 |
| RVEF (catheter) (%) | 52.4 (44.4–78.9) | 54.8 (51.7–68.8) | 51.8 (31.9–78) | 0.292 |
| RVEF (MRI) (%) | 59 (48–80) | 59.5 (32–61) | 24.5 (1–83) | 0.674e-6 |
| Grade of TR | 1.5 (0.25–3) | 1.5 (0–2) | 0.25 (0–3) | 0.876e-3 |
| PaO2 (mmHg) | 96.2 (48–112.7) | 85.8 (70.8–99.4) | 76.6 (59.2–90.6) | 0.164e-3 |
| PaCO2 (mmHg) | 39 (31.9–46.1) | 36.8 (32–41.1) | 38.3 (26–50) | 0.431 |
| AaDO2 (mmHg) | 4.0 (–10.4–61.8) | 19.7 (–1.0–36.8) | 25.7 (9.4–106.7) | 0.880e-3 |
| AST (U/L) | 27 (16–45) | 29 (17–37) | 31 (18–64) | 0.312 |
| ALT (U/L) | 20 (13–32) | 14 (11–19) | 22 (9–82) | 0.0169 |
| Albumin (g/dL) | 4.8 (4.3–5.2) | 4.3 (4.2–4.5) | 4.66 (3.8–5.6) | 0.0607 |
| Total bilirubin (mg/dL) | 0.9 (0.3–1.5) | 0.8 (0.2–1.2) | 0.7 (0.3–3) | 0.85 |
| Creatinine (mg/dL) | 0.49 (0.24–0.91) | 0.4 (0.3–0.56) | 0.49 (0.24–0.95) | 0.674 |
| Plt (×104/μL) | 25.6 (14.1–38.3) | 26.1 (22.3–38.5) | 23.5 (6.4–39.4) | 0.106 |
| BNP (pg/mL) | 20.6 (5.8–120.6) | 6.6 (0–13.3) | 10.4 (0–164.5) | 0.557e-2 |
| Peak VO2 (mL/kg/min) | 35 (29.7–39.6) | 23.2 (19.8–34.3) | 30.2 (18.9–53.9) | 0.174 |
| Reoperation (cases/%) | 7 (44) | 1 (16) | 1 (1.4) | 0.989e-5 |
| Arrhythmia (cases/%) | 5 (31) | 0 | 16 (22) | 0.352 |
| NYHA class (I/II/III) (cases) | 11/5/0 | 6/0/0 | 65/7/0 | 0.0638 |
Values are expressed as the median (range) except for those related to arrhythmias and reoperations. For arrhythmias and reoperations, the number of cases and corresponding percentages are presented. AaDO2: Alveolar-arterial oxygen difference, AscAo: Ascending aorta, AST: Aspartate aminotransferase, ALT: Alanine aminotransferase, BNP: Brain natriuretic peptide, BVR: Biventricular repair, CVP: Central venous pressure, LVEDP: Left ventricle end diastolic pressure, NYHA: New York Heart Association, PaCO2: Partial pressure of arterial carbon dioxide, PaO2: Partial pressure of arterial oxygen, Peak VO2: Peak oxygen consumption, Plt: Platelet count, RVEDV: Right ventricle end diastolic volume, RVEF: Right ventricle ejection fraction, SaO2: Arterial oxygen saturation, MRI: Magnetic resonance imaging, TR: Tricuspid valve regurgitation, PAWP: Pulmonary artery wedge pressure, SVCP: Superior vena cava pressure
Comparisons among the three groups revealed significant differences in age, SaO₂, CVP, SVCP, PAWP, and RVEDV (catheter, MRI), RVEF (MRI), grade of TR, PaO₂, AaDO₂, ALT, brain natriuretic peptide (BNP), and reoperation [Table 1]. Patients in the one-and-a-half group were significantly older than those in the Fontan group (P = 0.023), but there was no significant age difference between the BVR group and the other groups.
The CVP was significantly higher in the Fontan group than in the one-and-a-half group. No significant differences in CVP were observed between the BVR group and the other groups. In addition, in an analysis excluding one outlier case and limited to patients ≥ 6 years of age (BVR, n = 9; one-and-a-half, n = 4), no significant difference in CVP between the BVR and the one-and-a-half repair groups was observed (BVR: 5.67 ± 2.39 mmHg; one-and-a-half: 3.2 ± 1.48 mmHg; P = 0.15). SVCP was significantly lower in the BVR group than in the other groups. However, no significant difference in SVCP was found between the Fontan repair and the one-and-a-half repair groups [Figure 2].
Figure 2.
Central venous pressure (CVP) and superior vena cava pressure (SVCP) in the three groups (biventricular repair [BVR], one-and-a-half repair, and Fontan). The CVP was the highest in the Fontan group, and there was no significant difference between the BVR and the one-and-a-half repair groups. The SVCP was the lowest in the BVR group, and there was no significant difference between the one-and-a-half repair and Fontan groups. BVR: Biventricular repair, CVP: Central venous pressure, SVCP: Superior vena cava pressure
RVEF was significantly lower in the Fontan group than in the BVR (P = 0.018e-4) and one-and-a-half groups (P = 0.05), but there was no significant difference between the BVR and one-and-a-half groups. There were no significant differences in SaO₂, PaO₂, or AaDO₂ between the BVR and one-and-a-half groups. AaDO₂ was lowest in the BVR group and highest in the Fontan group, but no significant differences were observed between the one-and-a-half repair group and the other groups.
BNP also differed significantly among the groups; BNP in the BVR group was significantly higher than in the Fontan group, but no significant differences were observed between the one-and-a-half group and the other groups [Figure 3].
Figure 3.
Brain natriuretic peptide (BNP) and alveolar-arterial oxygen difference (AaDO2) in the three groups (biventricular repair [BVR], one-and-a-half repair, and Fontan). BNP was significantly lower in the Fontan group than in the BVR group, but there was no difference between the one-and-a-half repair and the Fontan groups. AaDO₂ was highest in the Fontan group, and decreased in the order of the one-and-a-half group and the BVR group. AaDO2: Alveolar-arterial oxygen difference, BNP: Brain natriuretic peptide, BVR: Biventricular repair
The reoperation rate was the lowest in the Fontan group and the highest in the BVR group. Reoperations in the BVR group included right ventricular outflow tract reconstruction (RVOTR) (n = 5), tricuspid valve (TV) plasty (n = 3), and RV overhaul (n = 3). The RV overhaul procedure included repeat pulmonary valvotomy, transatrial and transpulmonary resection of the hypertrophied infundibular muscle, and adjustment of interatrial communication.[3] No cases of arrhythmia were observed in the one-and-a-half group, whereas arrhythmias were most frequent in the BVR group. New York Heart Association (NYHA) functional class tended to be lower in the one-and-a-half group than in the other groups [Table 1]. In addition, all patients in the one-and-a-half repair group were classified as NYHA class I, whereas those in the other groups were classified as NYHA class II or III.
Figure 4 shows a scatter plot of CVP versus RVEDV for all patients. Using an RVEDV of 60% of normal as the cutoff value, the Fontan and BVR groups were clearly separated. However, the one-and-a-half group included patients both above and below this threshold. The distributions of the BVR and one-and-a-half groups overlapped considerably, and CVP varied widely among individual patients.
Figure 4.
Scatter plots showing the relationship between central venous pressure and right ventricular end-diastolic volume (RVEDV). In the Fontan group, the RVEDV was <60% of the normal RVEDV size, whereas in the biventricular repair and one-and-a-half repair groups, the RVEDV was more than 60% of the normal RVEDV size in most cases. BVR: Biventricular repair, CVP: Central venous pressure, RVEDV: Right ventricular end-diastolic volume
Figure 5 shows the reoperation-free rate for each repair group in this study. The horizontal axis represents the number of years since the final repair surgery. Over time, the BVR group had the lowest reoperation-free rate, followed by the one-and-a-half and the Fontan groups.
Figure 5.
Comparison of the reoperation-free rates between the three groups using Kaplan–Meier curves. The biventricular repair group had the lowest reoperation-free rate among the three groups, which was significantly different from that of the Fontan group (P = 0.0007). BVR: Biventricular repair
Among the eight deceased cases, one death occurred during the late postoperative period following Fontan repair, while the remaining seven occurred after the shunt procedures. The median age at death for these seven patients was 5 months (range: 1 month–3 years and 4 months), with five of the seven patients dying during infancy. The causes of death included heart failure due to a high pulmonary blood flow (n = 1), shunt failure (n = 2), myocardial ischemia (n = 1), and arrhythmia (n = 1). The remaining two patients died during early childhood (at 1 and 2 years of age, respectively). The 1-year-old patient had multiple congenital anomalies and died of respiratory failure due to aspiration pneumonia. The 2-year-old patient had coexisting aortic stenosis and died of pulmonary congestion due to a high pulmonary blood flow and diastolic dysfunction. The patient who died after Fontan completion was 3 years of age and died of multiple organ failure caused by infection. Seven of the eight deceased patients had coronary artery fistulas.
DISCUSSION
PAIVS is a rare congenital cardiac anomaly with an overall incidence of 4.5 cases/100,000 live births.[5] The clinical presentation, therapeutic strategy, and prognosis vary widely depending on the morphology of pulmonary atresia, degree of RV hypoplasia, TV morphology, and severity of TR.[6] Treatment strategies differ significantly across institutions, making multicenter studies difficult. Furthermore, few single-center studies have compared the long-term outcomes among different types of surgical repair.
The standard therapeutic strategy for PAIVS is to pursue BVR when sufficient RV growth and function are expected, and to consider either a one-and-a-half repair or a Fontan repair when biventricular circulation is deemed unachievable.[1,3,7] One-and-a-half repair is traditionally considered the second-best option when BVR is attempted but has proven unfeasible. This approach is based on the belief that BVR is superior to either the one-and-a-half repair or the Fontan repair because it involves physiological circulation. Patient selection for BVR is typically based on the RVEDV and TV diameter. When both parameters are ≤50% of normal, Fontan repair is generally selected; if RVEDV is ≥70% of normal and the TV diameter Z score is >-2, BVR is preferred. For intermediate cases, one-and-a-half repairs are considered.[8]
Historically, the surgical strategy for PAIVS at our institution involved a binary choice between BVR and Fontan repair. Although explicit numerical criteria were not defined, the general policy was to select BVR when RVEDV was approximately ≥60% of normal, and there were no significant issues with the TV; all other cases were directed toward Fontan repair. One-and-a-half repair was viewed as a palliative fallback for instances in which BVR was attempted but ultimately not feasible, rather than as a primary surgical goal. At the time, long-term complications following Fontan repair[9,10] were not well recognized. Owing to lower operative risks and fewer short-term reinterventions, Fontan repair was actively selected.
However, with the recent elucidation of long-term Fontan-related complications[9,10] and the increasing recognition that residual right-sided lesions (such as in adults with repaired tetralogy of Fallot) adversely affect prognosis,[11] our institutional perspective has evolved. We are now reconsidering our previous strategy by applying stricter RVEDV criteria and more actively debating the selection of a one-and-a-half repair rather than forcibly pursuing BVR.
Among patients with PAIVS who underwent surgical intervention at our institution, no late deaths were observed in the one-and-a-half or BVR groups. However, the BVR group had the highest reoperation rate. This suggests that reoperation is often essential in BVR patients, as commonly performed procedures (e.g. RVOTR and TV repair) aim to reduce RV load to prevent irreversible myocardial damage or to secure adequate volume to promote RV growth via RV overhaul. In the field of adult congenital heart disease, a greater number of reoperations is reportedly associated with poorer long-term outcomes,[12,13] and this inevitable need for further interventions represents a major drawback of the BVR strategy.
According to previous single-center reports, the rates of freedom from reintervention at 20 years in the BVR group and at 15 years in the one-and-a-half repair group were 75% and 69%, respectively, which was not statistically significant.[2,14] These studies also reported that the postoperative cardiac index, right atrial pressure, and exercise tolerance (as assessed by anaerobic threshold) in the one-and-a-half group were equivalent to those in the Fontan group, indicating suboptimal outcomes with no postoperative RV growth observed. In their cohort, the preoperative RV volume in the one-and-a-half group was relatively small (47 ± 23% of normal), which may have contributed to the higher reintervention rate.
In contrast, all patients in the one-and-a-half group at our institution remained NYHA class I, had fewer reoperations than the BVR group, and required no conversion procedures [Table 1]. Excluding one case with an RVEDV of 26% of normal, long-term CVP remained low, and the RV function was preserved. These findings underscore that the long-term risk of right heart failure is strongly correlated with the RV reserve and that BVR cannot be definitively considered superior to one-and-a-half repair.
Some centers advocate aggressive BVR for patients with TV diameter Z-scores ≥−3;[3] however, even in these cases, late conversion to one-and-a-half repair has been reported.[1,3] Such outcomes may reflect elevated CVP resulting from the selection of BVR in patients with a small RVEDV. Although delaying the final repair to allow RV growth is common, the occurrence of late conversion raises concerns regarding the predictability of RV development and long-term outcomes. Therefore, strict evaluation of RV volume at the time of final repair is imperative, and BVR should be considered only in patients with sufficient RV reserve. In patients with a borderline RV volume, one-and-a-half repair may be a more appropriate alternative. Furthermore, in our cohort, patients who underwent one-and-a-half repair with an RVEDV of 26% of normal had CVP levels equal to or higher than those of Fontan patients, suggesting they may have been better suited for Fontan repair. This finding reinforces the notion that the RV volume plays a crucial role in determining an appropriate repair strategy.
Achieving a favorable long-term RV function also depends on perioperative factors. Sugitani et al. reported that in BVR patients, pulmonary regurgitation and the RV/right atrial volume ratio 1 year after balloon pulmonary valvotomy significantly influenced late adverse events.[15] Given the fibrotic changes and diastolic dysfunction common in patients with PAIVS,[15] a comprehensive RV assessment is essential. Moreover, it has been reported that conversion to one-and-a-half repair does not necessarily improve restrictive RV physiology once right heart failure has occurred in BVR.[1] Therefore, we should avoid the idea that conversion from BVR to one-and-a-half repair is sufficient when heart failure occurs. The concept of promoting RV growth should be reconsidered, and the safety margin should be considered in the initial surgical planning. Reoperation is a poor prognostic factor,[12,13] and even if frequent reoperations are required after BVR to maintain circulation, which is barely established in infancy, long-term success may not be ensured.
Surgical decision-making in PAIVS is further complicated by factors beyond RV volume, including TV diameter and morphology, and the presence of coronary artery fistulas.[16] While some studies proposed selection criteria combining the TV diameter and RV volume,[17] qualitative aspects of valve morphology, which influence CVP, are also critical. In our study, the TR grade was comparable between the BVR and one-and-a-half groups, and the CVP was lower in the one-and-a-half group, but there was no statistically significant difference in CVP between the BVR and one-and-a-half groups. With the acquisition of additional case data in the future, it will be necessary to examine which factor, namely the grade of TR or RV preload, affects CVP more.
Coronary fistulas observed in most of the deceased patients in our cohort also represent a significant risk factor.[18] In a previous multicenter cohort study, coronary ischemia was the most frequent presumed mechanism of death, and the mortality rate was reported to be 8%,[18] which was similar to that observed in our study. The presence of RV-dependent coronary circulation often necessitates Fontan repair and is associated with unpredictable complications,[9,10,19] reinforcing the importance of optimizing RV reserve.
In their multicenter study, Iliopoulos et al.[19] reported that, although the cumulative incidence of events in the early postoperative period was lowest in the Fontan group, it increased over time, with the Fontan group showing the highest incidence among the three groups after 4 years postoperatively. These outcome differences can be explained by the organ responsible for driving the pulmonary circulation. In Fontan patients, pulmonary blood flow is driven by systemic venous pressure in the absence of the pulmonary ventricle, which results in fewer arrhythmias or reinterventions but a greater risk of systemic organ damage (e.g., Fontan-associated liver disease) due to elevated CVP. BVR restores physiological circulation but burdens the hypoplastic RV with pulmonary circulation. Without an adequate RV function, the risk of RV failure and arrhythmias increases. In contrast, one-and-a-half repair reduces the RV workload by diverting the upper-body venous return directly to the pulmonary arteries, thereby reducing the risk of right heart failure and arrhythmia compared with BVR.
Our findings support this physiological rationale: BNP levels differed significantly across groups. BNP was significantly lower in the Fontan group than in the BVR group, but there was no difference between the one-and-a-half and Fontan groups, suggesting higher RV wall stress in the BVR group. AaDO₂ was highest in the Fontan group, and decreased in the order of the one-and-a-half group and the BVR group, reflecting right-to-left shunting and systemic venous collaterals [Figure 3]. PaO₂ and SaO₂ were highest in the BVR group, implying superior oxygen delivery, which was also reflected in a trend toward higher peak VO₂.
However, SVCP was highest in the one-and-a-half group, raising concerns about complications (e.g. chylothorax and plastic bronchitis).[20,21] To mitigate elevated SVCP, we performed additional pulmonary artery banding on the side of the Glenn anastomosis to maintain adequate Glenn flow. As a result, although SVCP was higher than in BVR, it was comparable to that in Fontan patients, and all patients in the one-and-a-half group remained in NYHA class I. There were no SVCP-related complications, consistent with previous reports suggesting favorable outcomes with one-and-a-half repair despite an elevated SVCP.[21]
Limitations
This study was associated with several limitations. First, some bias may have resulted from the study’s retrospective design. Therefore, the findings may not be fully generalizable to future decision-making. Second, as this study was conducted at a pediatric hospital, adult hemodynamic indices could not be assessed. Although the one-and-a-half repair group had a median final follow-up age of 28.5 years, the follow-up durations for the other two groups were even shorter, and none of the patients had reached full adulthood; their anthropometric development was still ongoing. Consequently, the future trajectory of SVCP and related complications in adulthood remains unclear. Third, the effects of relatively elevated SVCP on cerebral circulation and, thus, neurodevelopmental outcomes were not investigated in this case series. Fourth, the single-center design introduced inherent bias, and the limited number of one-and-a-half repair cases reduced the statistical power, underscoring the need for further case accumulation and ongoing investigation.
CONCLUSIONS
PAIVS is a congenital heart disease characterized by RV hypoplasia that confers a high risk of RV failure with advancing age. Although BVR restores physiological circulation, it imposes significant stress on the RV, resulting in the highest rates of reoperation and adverse events. In contrast, one-and-a-half repair offers a safer hemodynamic margin and may carry a lower risk of long-term RV failure than BVR. Given the importance of long-term outcomes, we advocate for the more stringent application of current RVEDV-based criteria to better identify patients suitable for BVR and to reduce late complications by carefully selecting the most appropriate surgical strategy.
Conflicts of interest
There are no conflicts of interest.
Funding Statement
Nil.
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